Scleroderma: Diagnosis, Treatment, and Steps to Take

Diagnosis of Scleroderma

It can be difficult for doctors to diagnose scleroderma because the symptoms vary from person to person and are similar to other diseases. There is no single test to diagnose the disease; instead doctors use a combination of the following to help diagnose scleroderma. Your doctor may:

• Ask about your medical history.
• Ask about your current and past symptoms.
• Perform a physical exam.

Your doctor may recommend additional testing such as:

• Ordering laboratory tests to check for certain antibodies that mistakenly target and react to your own tissues. Some of the antibodies may be common in people with scleroderma. However, antibodies may develop due to other factors, so a blood test alone does not diagnose scleroderma.
• Performing a skin biopsy.

To look for problems with internal organs, such as the heart, lungs, or kidneys, your doctor may order additional testing. Early diagnosis of organ involvement helps doctors treat and manage the disease. Testing may include:

• Computerized tomography (CT), which uses a scanner to take images of the lungs and other organs.
• Echocardiogram, which uses sound waves to create moving pictures of your heart.
• Pulmonary function tests, which measure the function of the lungs.

Treatment of Scleroderma

Your treatment depends on the type of scleroderma you have, your symptoms, and which tissues and organs are affected. Treatment can help control the symptoms and limit damage.

Your doctor may recommend medications, including:

• Anti-inflammatory medications to manage pain and reduce swelling.
• Topical creams to treat skin changes, including tightness and itching.
• Immunosuppressants, which may suppress the overactive immune system and can help control some aspects of the disease. Your doctor may prescribe oral, IV, or injectable immunosuppressants.
• Vasodilators to help blood vessels dilate (widen), which may treat Raynaud’s phenomenon and some lung issues.

In addition, your doctor may prescribe medications that are typically approved to treat other rheumatic diseases that have similar symptoms to scleroderma.

Many people benefit from physical or occupational therapy to:

• Relieve pain.
• Improve muscle strength and mobility, including muscles in your arms, legs, and jaw.
• Teach you techniques to help with activities of daily living. For example, if hand pain and stiffness make it hard to brush your teeth, a therapist can recommend toothbrushes and devices to make flossing easier.

Regular dental care is important because scleroderma can make your mouth dry and damage connective tissues in your mouth, speeding up tooth decay and causing your teeth to become loose. Tightening facial skin can also make your mouth opening smaller and narrower, which makes it harder to care for your teeth. Here are some ways to avoid tooth and gum problems:

• Brush and floss your teeth regularly.
• Have regular dental checkups. Contact your dentist immediately if you experience mouth sores, mouth pain, or loose teeth.
• Talk to your dentist and doctor about the best methods for you to use to keep your mouth moist.
• Use special mouthwashes or toothpastes for dry mouth. You can also talk to your doctor about medications that treat dry mouth.

Lung Damage

Almost all people with systemic scleroderma have some loss of lung function. Some people develop severe lung disease, which comes in two forms:

• Pulmonary fibrosis, a hardening or scarring of lung tissue because of excess collagen.
• Pulmonary hypertension, high blood pressure in the artery that carries blood from the heart to the lungs.

Treatment differs for these two conditions:

• Pulmonary fibrosis may be treated with medications that suppress the immune system, or medications which can help counter fibrosis.
• Pulmonary hypertension may be treated with medications that dilate the blood vessels.

To help minimize lung complications, work closely with your doctor.

• Watch for signs of lung disease, including fatigue, shortness of breath, dry cough, or difficulty breathing, and swollen feet. Report these symptoms to your doctor.
• Follow up regularly with your doctor for evaluation of your lung function. This may include standard lung function tests, which measure your lung volumes to monitor the course of lung fibrosis. Checking for pulmonary hypertension early helps doctors manage and treat the condition, even before you may notice symptoms.
• Get regular flu and pneumonia vaccines as recommended by your doctor, especially if you are taking immune-suppressing medications or have lung disease.

Heart Problems

Some people may develop complications that cause heart problems, including:

• Cardiomyopathy, scarring and weakening of the heart.
• Myocarditis, inflamed heart muscle.
• Arrhythmia, abnormal heartbeat.

Treatments for heart complications can range from medications to surgery and vary depending on the nature of the condition.

Kidney Problems

Renal crisis is uncommon but can be serious for people with systemic scleroderma. Renal crisis happens when blood pressure levels rise suddenly to dangerous levels, which can quickly lead to kidney failure. Side effects of certain medications, such as corticosteroids, can also trigger renal crisis. It is important that you and your doctor work together to monitor your blood pressure, including:

• Check your blood pressure regularly, and let your doctor know if you have any new or different symptoms such as a headache or shortness of breath. If your blood pressure is higher than usual, call your doctor right away.
• If you have kidney problems, remember to take your medications as prescribed. In the past several decades, medications known as ACE (angiotensin-converting enzyme) inhibitors have made scleroderma-related kidney failure a less threatening problem than it used to be.

Who Treats Scleroderma?

Most people will see a rheumatologist for scleroderma treatment. A rheumatologist is a doctor who specializes in rheumatic diseases such as arthritis and other inflammatory or autoimmune disorders. Dermatologists, who specialize in conditions of the skin, hair, and nails, may also play an important role in treating the disease, particularly for people with localized scleroderma.

Because scleroderma can affect many different organs and organ systems, you may have several different doctors providing your care. These health care providers may include:

• Cardiologists, who specialize in treating diseases of the heart and blood vessels.
• Dental providers, who can treat complications from the thickening of tissues of the mouth and face.
• Gastroenterologists, who treat digestive problems.
• Mental health professionals, who provide counseling and treat mental health disorders such as depression and anxiety.
• Nephrologists, who treat kidney disease.
• Occupational therapists, who teach how to safely perform activities of daily living.
• Orthopaedists, who treat and perform surgery for bone and joint diseases or injuries.
• Primary care providers, including physicians, nurse practitioners, and physician assistants.
• Physical therapists, who teach ways to build muscle strength.
• Pulmonologists, who treat lung disease and problems.
• Speech-language pathologists, who specialize in the treatment of speech, communication, and swallowing disorders.